Klippel-Trenaunay syndrome: isithombe, izimpawu, ukwelashwa

Okuningi izifo sebuningini kwakhiwa kwesikhathi sombungu. Ngakho-ke, le nomthelela ekukhuleni kwabo abakwazi njalo. Phela, iningi lalezi kwezinso uxilongwe kuphela ngemva kokuzalwa kwengane. Futhi kamuva abanye okuningi. Ukuze omunye nezifo ngenxa yezakhi zofuzo kuzomele sifake Klippel-Trenaunay isifo. Lesi sifo latholakala ekuqaleni kwekhulu lama-20. Lokhu kuthiwa kubangelwa ukuvela isayensi yezakhi zofuzo. Ngokuvamile lesi sifo kwabesilisa singangena. Nokho, ibuye yathola phakathi kwabantu besifazane. Pathology isibaluli azalwa naso iziyaluyalu semithambo.

ngalesi sifo, Klippel-Trenaunay Incazelo

Klippel-Trenaunay syndrome kubhekwa isici esingavamile esidluliselwa ngofuzo. Ukubikezela ukuthuthukiswa kwalesi zokugula cishe kungenakwenzeka, ngoba isici singesabo ezingavamile futhi hhayi kutholakala ukuthi uphethwe yini ngesikhathi ukhulelwe. Nokho, kwezinye izimo, isifo kungenziwa esolwa Klippel-Trenaunay. Lesi sifo libhekisela kuyisifo esiwufuzo. Ngakho-ke, ukuhlaziya zofuzo kumele babhekane abesifazane abakhulelwe emkhayeni lapho amacala lesi sifo iphutha. Enye igama isifo - hemangiectasia hypertrophic. Lwasemtholampilo isithombe Klippel-Trenaunay syndrome kuhlanganisa izimpawu ezintathu eziyinhloko:

1. varicose emithanjeni.
2. Ukubukeka yobudala izindawo ezihlala esikhumbeni.
3. Ukushintsha ububanzi nobude esitho ethintekile.

Kwezinye izimo of lwamathambo kanye ezithambile izicubu hypertrophy uyalandelwa. Ngokuvamile Pathology libhekene Yiqiniso ezithuthukayo. Onalesi sifo incike kobukhulu izinguquko semithambo. Nakuba imbangela isifo kungenakususwa, izinyathelo zokwelapha ukusiza ukuthuthukisa izinga lempilo isiguli. Abantu abaphethwe yilesi sifo inikezwa ukukhubazeka.

Izimbangela Klippel-Trenaunay syndrome

Ngesizathu esithile, kukhona isifo Klippel-Trenaunay? I syndrome ngenxa isici zofuzo. Lokhu anomaly idluliselwa i autosomal evelele ifa hlobo. Lokhu kusho ukuthi uma kunesici ezifana zofuzo kwelinye abazali, kukhona kungenzeka yalesi sifo ngo-ingane-50%. Futhi, lesi sifo angadluliselwa ngokusebenzisa phakathi nesizukulwane esisodwa, okungukuthi - kumkhulu nogogo nakubazali. Kulokhu, ematfuba 25%.

Njengazo zonke kwezinso zofuzo, ukukhubazeka semithambo ukuthuthukisa ngesikhathi fetus izicubu ibhukumaka. Ingalimaza imiphumela elimazayo. Lezi zihlanganisa izinto ezifana:

1. ukungakhululeki ngokwengqondo namasonto amabili okuqala okukhulelwa.
2. Izifo ezithathelwanayo. Lokubaluleke kakhulu kukhona izilonda ezingamahlalakhona yegciwane emzimbeni.
3. Ukusetshenziswa kwezidakamizwa kanye izakhiwo teratogenic.
4. Ukwamukelwa izidakamizwa nje kokuba akhulelwe ngesikhathi sokukhulelwa.
5. Ukubhema nokuphuza utshwala.
6. Ukungcoliswa komoya ngo ezinobuthi, emisebeni.

Zonke lezi zici zingabangela nezinguquko emzimbeni kwabesifazane kanye naye umntwana ongakazalwa. Ziyakwazi esiyingozi ikakhulu uma nisanda zokuqala zokukhulelwa. Ngemva anomalies ahlukahlukene kungenziwa elakhiwe kule nkathi.

Mechanism lesi sifo

Kungani kukhona izinguquko semithambo e isifo Klippel-Trenaunay? Lesi sifo ububonisa izimpawu kuphazamiseka emithambo yegazi. Mshini yentuthuko zokugula ngu uvimba outflow wegazi bevela emikhawulweni aphansi. Lokhu kungenxa yokuthi mafutha anciphisa isikhala esingaphakathi emithambo yegazi is ayisesemikhulu, kukhona kokucindezelwa kwegatsha isitsha ophakeme, kwemisipha hypertrophied, izicubu asalile sombungu. Ngenxa yokwephula ka outflow wegazi kukhona nomfutho wegazi ophezulu. Vienna kancane kancane thinned, wadalula umonakalo. Ngaphezu kwalokho, kwaphazamisa ukusebenza kwe-apharathasi valve. Ngenxa igazi stasis esitho uqala hypertrophy. Ngenxa yokujama tsi ahlupheka umsebenzi trophic. Ngakho-ke, izicubu wadalula ischemia. ukuphazamiseka engapheli eyayiqhutshwa kokuphakelwa kwegazi kuholela sclerosis. Soft izicubu hypertrophy kwenzeka hyperemia futhi edema. Kwezinye izimo, iyashintsha namathambo. Ngenxa yalokho ngamangala umlenze ungandi kuphela ububanzi, kodwa futhi ube mude.

Klippel Trenaunay Syndrome-: izimpawu

Ngu izenzo Klippel-Trenaunay syndrome zihlanganisa izinguquko hhayi kuphela yesimiso emithambo yegazi, kodwa amanye zamehlo. Naphezu kweqiniso lokuthi angahlangani azalwa naso, izimpawu zokuqala zingavela yobusha noma eminyakeni yokungena ebudaleni kukhombisa. Ngakho, izenzo zokwelapha zihlanganisa ukwephulwa lokhu okulandelayo:

1. Ukuvuleka komlomo olukha phezulu emlenzeni emithanjeni. Kubonisa kinking sokugembula nobuhlungu kanye isitsha angase abonwe nangeso lenyama. Ezimweni eziningi varicose emithanjeni esisogwini ebusweni elingaphandle imilenze.
2. Ukwanda inani zomzimba. Ngokuvamile ephelezelwa izenzakalo ukuvuvukala. Ngenxa igazi stasis umlenze abathintekayo zingase zihluke kakhulu kusukela esitho enempilo. Ngokuvamile kuba hyperemic, isikhumba uqweqwe.
3. Zokwenziwa kwelulwa. Hypertrophy can kokubili izinyawo nezandla.
4. Ukubukeka nevi esikhunjeni esikhumbeni. Ngenye indlela, kulezi zokwakheka abizwa ngokuthi "iwayini amabala".

Futhi, ne ukuqhubeka kwesifo avele izinguquko trophic. ukukhubazeka esidalwa yizilonda emathunjini sibe singenzeka abaningi babephephile ezindaweni ezithintekile, okuyinto musa ukuphulukisa okwesikhathi eside. Phakathi kwezinye kwezinso ekuthuthukiseni kule zokugula, kukhona polydactyly, khona tho iminwe noma izinzwane sixhumene.

Indlela ukuxilonga izifo Klippel-Trenaunay?

Syndrome Ukuxilongwa Klippel-Trenaunay uvame ukusekelwa ukubonakaliswa emtholampilo. Izimpawu zesifo avele kancane kancane. Uphawu lokuqala kungenzeka ukubonakala "iwayini amabala" noma ngemithambo. Uma lezi zimpawu ehlangana hypertrophy esitho wadalula uphethwe: Klippel-Trenaunay syndrome. Izithombe zabantu abaphethwe yilesi sifo zifana nomunye. Abakwazi ukubona izincwadi enikelwe pathologies esingavamile esidluliselwa ngofuzo. Ngakho-ke, uphethwe kakhulu akunzima.

Ngaphezu idatha emitholampilo, wenziwa venography, ultrasonography yezingalo nemilenze ephansi, ama-X ray.

Klippel Trenaunay Syndrome-: ukwelapha isifo

ukusebenza kubhekwa umcimbi kwezokwelapha. Umthamo kokungenela kunqunywa degree of lesion semithambo. Ukuze sandise mafutha anciphisa isikhala esingaphakathi we stenting vein wenziwa. Lapho endaweni enkulu nolokubhujiswa ukhiqiza dlula isitsha - esikhundleni salo kube nokuthile isayithi nolwelwesi.

Ukuze ulwe varicose skleroteropiya esimisiwe, laser coagulation emithanjeni. Iziguli ezinale isifo kumele bagqoke kokucindezelwa zangaphansi.

ngalesi sifo, Klippel-Trenaunay Prevention

Klippel-Trenaunay syndrome ezinsaneni kuyaqabukela kutholakala ukuthi uphethwe yini. Ngokuvamile-ke itholwa ezinyangeni zokuqala noma iminyaka yokuphila. Ukuvimbela isifo, kubalulekile ukuba bazivikele emiphumeleni elimazayo ngesikhathi sokukhulelwa. Esinye isilinganiso zokuvimbela ubhekwa ube luhlolo zofuzo umbungu ebukhoneni ezifo emkhayeni.